Fraser syndrome associated with anterior urethral atresia

Abstract

Except for hypospadias, congenital abnormalities of anterior urethra are rare [Karnak et al., 1995]. Obstructive abnormalities including valves and atresia are much less frequent than the posterior urethra defects.To the best of our knowledge, this is the first documented case of Fraser syndrome associated with atresia in the anterior part of the urethra and also it is the first time that atresia is reported in the anterior part of the urethra. Therefore, newborn infants with Fraser syndrome and patients with syndromes associated with urogenital anomalies, especially with umbilical discharge or severe hydroureteronephrosis, should be evaluated for the patency of the anterior urethra. ACKNOWLEDGMENTSThe authors are grateful to Prof. Dr. Sevim Balcı from the Department of Genetics for close collaboration. Fig. 2. The radiocontrast study obtained via the umbilical opening demonstrates the deformed urinary bladder, vesicoureteroreflux, the posterior urethra with normal calibration, and the atresia in the anterior urethra with cystic dilation behind. REFERENCES